Patient monocytes respond differently to NLRP3 priming and/or activation, and NLRP3 inhibitor MCC950 depending on NLRP3 variants. (A) Monocytes of 22 autoinflammatory patients and healthy donors (WT) were treated with LPS (40 ng/ml, 3 h) and nigericin (5 μg/ml) in the presence of MCC950 (1 μM, added 15 min before LPS), and cell death was monitored by PI incorporation over time quantified by time-lapse high content microscopy for 2 h. (B) R260W, E525K+D646Y, and K565E (group#5). (C) E311K and E690K (group#4). (D) T348M and A439V (group#3). (E) S726G+S896P, Q703K, and V198M (group#2). (F) T952M (group#1). Means of AUC of two to six replicates and 1 SD are represented. One experiment done in two to six replicates is shown. Data represented in the same graph correspond to one same experiment on relatives. Due to limited amount of blood from patients E525K/D646Y P1, K565E P1 and P2, and T348M P1, some conditions could not be tested.